Nuances in the surgical treatment of gangliocytomas of the spinal cord: illustrative cases.

BACKGROUND

Intramedullary gangliocytoma, a tumor rarely seen in the spinal cord, presents unique diagnostic and operative challenges. The authors present 2 cases illustrating these complexities.

OBSERVATIONS: The first patient, a 34-year-old woman with cerebral palsy, had 6 years of ascending numbness and worsening leg weakness, bladder incontinence, and thoracic radicular pain. MRI revealed a slightly enhancing area of cord enlargement extending from T5 to the conus. The second patient, a 45-year-old man with unilateral vestibular schwannoma, presented with slowly progressive left leg weakness and an intramedullary tumor at C7-T1. Each patient underwent surgical tumor removal, with one resected completely and the other subtotally. Both surgical specimens demonstrated clusters of ganglion cells strongly immunopositive for neurofilament proteins amid prominent neuropil islands, with no evidence of a glial component, confirming a diagnosis of gangliocytoma. The first patient had a follow-up period of 13 years without tumor recurrence, while the second patient had a follow-up of 3 months.

LESSONS: Although these tumors have a pseudocapsule that may assist safe resection, their interface with normal cord is sometimes focally indistinct. Subtotal resection in some cases may prevent new neurological deficit while permitting long-term maintenance of preoperative levels of function with stable residual disease. https://thejns.org/doi/10.3171/CASE2570.